Kaddu S, Beham A, Cerroni L, Humer-Fuchs U, Salmhofer W, Kerl H, Soyer H P
Department of Dermatology, University of Graz, Austria.
Am J Surg Pathol. 1997 Sep;21(9):979-87. doi: 10.1097/00000478-199709000-00001.
We report the clinical, histopathologic, immunohistologic, and prognostic findings in 19 patients with cutaneous leiomyosarcoma, eight males and 11 females (mean age, 66 years; age range, 41-93 years). The tumors presented mainly as solitary lesions and were located on the head and neck (eight lesions), trunk (four lesions), upper extremities (three lesions), and lower extremities (four lesions). Histopathologically, two predominant growth patterns were observed: nodular (12 cases) and diffuse (seven cases). Neoplasms with a nodular growth pattern were characterized by high cellularity and prominent nuclear atypia, and they showed conspicuous mitoses, several necrotic cells, and sometimes extensive necrotic areas. By contrast, most cutaneous leiomyosarcomas with a diffuse growth pattern revealed low cellularity, well-differentiated smooth muscle cells, inconspicuous mitotic figures, and few or no necrotic cells. Immunohistologic investigations revealed all cutaneous leiomyosarcomas to express vimentin and smooth muscle actin. Pan-muscle actin (HHF-35) was also expressed in most cases (15 lesions). However, only 12 lesions showed positive staining for desmin. Remarkable was the expression of cytokeratins in five lesions. Clinical follow-up revealed local recurrences in five patients (three cases with nodular pattern and two lesions with a diffuse pattern) after a period ranging from 8 months to 3 years after surgical excision. No distant metastases have been observed in our series. We conclude that cutaneous leiomyosarcoma with a diffuse growth pattern may constitute a pitfall in histopathologic diagnosis because of the presence of only subtle criteria for malignancy. Cutaneous leiomyosarcoma may show different immunophenotypes, thus emphasizing the importance of using a large panel of antibodies (smooth muscle actin, HHF-35, desmin, vimentin, cytokeratins, and S-100 protein) in immunohistologic diagnosis. Cutaneous leiomyosarcoma sometimes reveals local recurrences, but it has negligible potential for distant metastases.
我们报告了19例皮肤平滑肌肉瘤患者的临床、组织病理学、免疫组织化学及预后情况,其中男性8例,女性11例(平均年龄66岁;年龄范围41 - 93岁)。肿瘤主要表现为孤立性病变,位于头颈部(8处病变)、躯干(4处病变)、上肢(3处病变)及下肢(4处病变)。组织病理学上,观察到两种主要生长模式:结节状(12例)和弥漫性(7例)。具有结节状生长模式的肿瘤细胞密度高,核异型性显著,有明显的核分裂象、数个坏死细胞,有时还有广泛的坏死区域。相比之下,大多数具有弥漫性生长模式的皮肤平滑肌肉瘤细胞密度低,平滑肌细胞分化良好,核分裂象不明显,坏死细胞很少或没有。免疫组织化学研究显示,所有皮肤平滑肌肉瘤均表达波形蛋白和平滑肌肌动蛋白。大多数病例(15处病变)也表达泛肌动蛋白(HHF - 35)。然而,仅12处病变结蛋白染色呈阳性。值得注意的是,5处病变中有细胞角蛋白表达。临床随访发现,5例患者在手术切除后8个月至3年期间出现局部复发(3例为结节状模式,2处病变为弥漫性模式)。在我们系列病例中未观察到远处转移。我们得出结论,具有弥漫性生长模式的皮肤平滑肌肉瘤在组织病理学诊断中可能是一个陷阱,因为其恶性标准仅很细微。皮肤平滑肌肉瘤可能表现出不同的免疫表型,因此强调了在免疫组织化学诊断中使用一大组抗体(平滑肌肌动蛋白、HHF - 35、结蛋白、波形蛋白、细胞角蛋白和S - 100蛋白)的重要性。皮肤平滑肌肉瘤有时会出现局部复发,但远处转移的可能性极小。